systemic sclerosis

Question 1

definition

Answer 1

systemic connective tissue disease characterised by vasomotor disturbances (raynauds) and fibrosis and subsequent atrophy of the skin

Question 2

who is systemic sclerosis more common in

Answer 2

4x more common in females and those in their 40s and 50s

Question 3

pathophysiology

Answer 3

• T cells infiltrate the skin and there is abnormal fibroblast activation leading to increased production of the ECM in the dermis causing sclerodactyly
• arterial and arteriolar narrowing occurs due to intimal proliferation and vessel wall inflammation causing increased release of platelets and vasoconstrictors causing ischaemia

Question 4

subdivided into

Answer 4

limited and diffuse systemic sclerosis

Question 5

limited systemic sclerosis

Answer 5

skin involvement confined to face hands, forearms and feet

Question 6

limited systemic sclerosis was previously called

Answer 6

CREST which stands for 
Calcinosis 
Raynuads
Esophageal dysmotility 
Sclerodacty
Telangiectasia

Question 7

antibody for limited systemic sclerosis

Answer 7

anti-centromere antibody

Question 8

diffuse systemic sclerosis

Answer 8

skin involvement of face, arms, forearms, hands and torso

Question 9

5 year survival rate of diffuse systemic sclerosis

Answer 9

70%

Question 10

antibody in diffuse systemic sclerosis

Answer 10

anti-scl-70 antibody

Question 11

associated problems of both types of systemic sclerosis

Answer 11

• pulmonary hypertension
• intestinal bacterial overgrowth
• interstitial fibrosis
• renal crisis triggered by malignant hypertension

Question 12

general management of systemic sclerosis

Answer 12

• raynauds treat with NIFEDIPINE
• oesophageal dysmotility treat with OMEPRAZOLE
• bacterial overgrowth treat with ANTIBIOTICS
• pulmonary hypertension treat with ENDOTHELIN ANTAGONIST, HOME O2, PROSTACYCLIN
• renal involvement treat with ACE INHIBITORS
• interstitial fibrosis treat with CYCLOPHOSPHAMIDE